I'm not sure how much material has been published. Much of the world experience comes from places where access to formulas for life is not an issue. Phillip Lee in London runs a clinic for adults with IEMs--he would probably be able to give you a sense of how many of his patients with MMA, etc can get along on just a low-protein diet, and how many need medical food. My guess is that only the urea-cycle people will be able to get along on just low-protein diet. In the past 8 years I've worked in Australia and Maryland, where access to medical foods was not restricted at age 21. Our older patients were all on medical foods, except for the PKU patients who didn't want to be (most did). In Arkansas we have the opposite situation--people who want to be on the PKU diet, but can't afford it (who can?) and don't have insurance. The 'safety net' doesn't really catch them--the result is high PHE levels and poor brain functioning, which will be progressively harder to treat. A cost-benefit analysis would be hard to do, and there will be a lot of wobble in the figures, but it might be a useful exercise. Long-term consequences will be very hard to figure--progressive renal damage in MMA, osteopenia/porosis with chronic acidosis and poor intake, learning problems, diminished executive/frontal lobe functioning, psychological problems that have a biochemical basis, etc. It's been hard to figure this for PKU, so I'm sure it will be more difficult for rarer disorders. You might want to submit your question to/through the metab-l listserve. If you don't have access or someone local who will do this for you, I'll be happy to be an intermediary. Steve Stephen G. Kahler, MD Professor, Division of Clinical Genetics Department of Pediatrics University of Arkansas for Medical Sciences Email:[log in to unmask] Office Phone 501-364-2966 Fax 501-364-1564 Pager (alphanumeric) 501-395-7865 Mail:Division of Clinical Genetics Slot 512-22 Arkansas Children's Hospital 800 Marshall St. Little Rock, AR 72202-3591 ======================================================================== ======================== Confidentiality Notice: This e-mail message, including any attachments, is for the sole use of the intended recipient(s) and may contain confidential and privileged information. Any unauthorized review, use, disclosure or distribution is prohibited. If you are not the intended recipient, please contact the sender by reply e-mail and destroy all copies of the original message. ======================================================================== ======================== -----Original Message----- From: Communication for the Heartland Regional Genetics Group [mailto:[log in to unmask]] On Behalf Of Troxell, Robin M. Sent: Monday, April 24, 2006 12:11 PM To: [log in to unmask] Subject: FW: cost-benefit analysis of providing metabolic formula From: Troxell, Robin M. Sent: Mon 4/24/2006 10:03 AM To: [log in to unmask]; Heartland, Chandra Hope (UMC-Student) Subject: cost-benefit analysis of providing metabolic formula Dear all - Does anyone have a good article on the need for individuals on metabolic formulas to stay on them for life, focusing not on PKU (there are many articles on that). Specifically, the risk for MR/ health problems when adults lose their insurance coverage and cannot afford the formula. Thanks. Robin ********************************* Robin M. Troxell, MS, CGC Genetic Counselor University of Missouri Department of Child Health Mercy St. John's Hospital (p) 417-820-9839 (f) 417-820-3720 [log in to unmask] ================================================================================================ Confidentiality Notice: This e-mail message, including any attachments, is for the sole use of the intended recipient(s) and may contain confidential and privileged information. Any unauthorized review, use, disclosure or distribution is prohibited. If you are not the intended recipient, please contact the sender by reply e-mail and destroy all copies of the original message. ================================================================================================